Korean J Gastroenterol.  2014 Mar;63(3):176-182. 10.4166/kjg.2014.63.3.176.

A Case of IgG4-Related Sclerosing Mesenteritis Associated with Crohn's Disease

• Kim EJ
• Kim EY
• Song JE
• Lee HC
• Bae GH
• Oh HK
• Lee TS

• KMID: 1730929
• DOI: http://doi.org/10.4166/kjg.2014.63.3.176

Abstract

Sclerosing mesenteritis (SM) is a rare disease characterized by chronic nonspecific mesenteric inflammation and fibrosis of unknown etiology. Some tumefactive SM shows diffuse accumulation of IgG4-positive plasma cells and is considered as a part of the spectrum of IgG4-related disease. An association between inflammatory bowel disease and IgG4-related disease has been indicated. A 45-year-old woman visited our hospital due to weight loss with intermittent lower abdominal discomfort. Pelvic ultrasound revealed a mass-like lesion in the abdominal wall and pelvis MRI demonstrated a 5.9 cm sized wall-enhancing mass with heterogeneous signal intensity from right adnexa to the abdominal wall. Tumor resection and adhesiolysis was done because of severe adhesion with the small bowel, colon, bladder, uterus, and abdominal wall. Appendectomy was also performed due to adhesion and edematous change. Histological examination of the resected mass showed findings that were compatible with IgG4-related SM. The resected appendix showed chronic granulomatous inflammation without evidence of tuberculosis. She was diagnosed with Crohn's disease after undergoing colonoscopy and CT enterography. Herein, we report a rare case of IgG4-related SM that occurred in conjunction with Crohn's disease.

Keyword

Sclerosing mesenteritis; IgG4-related disease; Crohn disease

MeSH Terms

Anti-Inflammatory Agents/therapeutic use
Appendix/pathology
Azathioprine/therapeutic use
Colonoscopy
Crohn Disease/complications/*diagnosis/drug therapy
Female
Humans
Immunoglobulin G/*blood
Magnetic Resonance Imaging
Mesalamine/therapeutic use
Middle Aged
Panniculitis, Peritoneal/*diagnosis/etiology/ultrasonography
Prednisolone/therapeutic use
Tomography, X-Ray Computed
Urinary Bladder/pathology
Anti-Inflammatory Agents
Azathioprine
Immunoglobulin G
Mesalamine
Prednisolone

Figure

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  Fig. 1. Pelvis MRI findings. Axial (A) and sagittal (B) view of contrast-enhanced fat-saturated T1-weighted images show a 5.9 cm sized wall-enhancing mass (arrows) with heterogeneous signal intensity from right adnexa to the abdominal wall.

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  Fig. 2. Histologic findings of mesenteric mass. (A) Low magnification of mesenteric lesion shows marked fibrosis and inflammatory cell infiltration (H&E, ×10). (B) The inflammatory infiltrates are polymorphic with numerous plasma cells (H&E, ×400). (C, D) Many IgG4 expressing plasma cells are noted in the stoma around the fat lobule (IgG4, ×400).

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  Fig. 3. Histologic findings of appendix and urinary bladder. (A) Transmural inflammation and small noncaseating granulomas are seen in the appendiceal mucosa (H&E, ×20). However, IgG4 expressing plasma cells are absent in the specimen from (B) appendix (IgG4, ×400) and (C) urinary bladder (IgG4, ×200).

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  Fig. 4. Colonoscopic findings. (A, B) Colonoscopy shows active ulcers, pseudopolyps and ulcer scars on cecum and ileocecal valve. (C, D) Numerous aphthous ulcers are scattered throughout the entire colon and rectum.

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  Fig. 5. Histologic findings of colonoscopic biopsy specimen. Colon mucosa shows small granuloma (circle) and inflammatory infiltration with erosion (H&E, ×100).

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  Fig. 6. CT enterography finding. Axial CT scan shows irregular wall thickening with edema along the distal ileum and mesenteric infiltration (arrow).

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  Fig. 7. Follow-up colonoscopic findings. Multiple variable sized ulcers with polypoid lesions are noted at terminal ileum (A) and ileocecal valve (B). Multiple aphthous erosions are scattered on sigmoid colon (C).

Reference

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